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Biomedical subjects

C Bernoulli

Publications and source records attributed to C Bernoulli.

13 recordsLinked to original sources
PubMed1998

Biotin determination by three different methods: specificity and application to urine and plasma ultrafiltrates of patients with and without disorders in biotin metabolism.

A microbiological, an avidin-binding and a streptavidin-binding method for biotin determination were compared. All three methods detected biotin equally well but they exhibit different specificities for derivatives of biotin. The microbiological assay has the highest specificity and is the method of choice for biotin determination in biotinidase-deficient patients. The specificity of streptavidin-binding has not been investigated so far. Application of the three methods to urine samples of patients with and without biotin therapy indicated that only 50% of biotin equivalents measured with the avidin method correspond to authentic biotin as previously shown. The other 50% comprise mainly bisnorbiotin and biotin-d-sulfoxide. HPLC-separation of urine samples prior to assay confirmed this finding and revealed a bisnorbiotin oxidation product and an unknown compound as further biotin metabolites. The latter was measurable by all three methods and not detectable in plasma ultrafiltrate. This was the only metabolite which was able to restore deficient 3-methylcrotonyl-CoA carboxylase activity in biotin-deficient fibroblasts. The combination of the three methods together with HPLC-separation proved to be a valuable analytical tool for the identification of the main biotin metabolites in biological fluids.

Administration, Oral↗
PubMed1980

Clinical and chronotopographic psychomotor seizure patterns (SEEG study with reference to postoperative results).

In 29 selected patients who underwent stereo-electroencephalographic exploration because of uncontrolled psychomotor epilepsy, 213 seizures were recorded. These seizures were analyzed in 10-second-intervals with respect to the sequence of clinical signs and the electrical chronotopographic patterns. By the aid of a computerized cluster analysis four comparatively distinct localization-patterns were found: a) opercular, b) frontobasal-cingular, c) temporobasal-limbic and d) posterior temporal neocortical. The lowest trend for propagation to the opposite hemisphere is found in a) followed by d). Fairly strong tendency for contralateral propagation is seen in c). Strategically important structures for contralateral propagation are: 1. amygdala and hippocampus, and to a lesser degree 2. the frontal cortex. Seizures propagating to the parietal cortex very often involve the frontal area of the same side before affecting the opposite hemisphere. For each of these "epileptic clusters" the characteristic clinical signs are pointed out. Special emphasis was put on the primictal symptom-sequence. By this and a similar study with Bancaud et al. we show that significantly better postoperative results are found, when strategy for surgery was based on the evidence of several SEEG recorded spontaneous seizures. In some patients belonging to the "temporobasal-limbic" cluster stereotactic procedures proved successful.

Amygdala↗
PubMed1979

Creutzfeldt-Jakob disease: patterns of worldwide occurrence and the significance of familial and sporadic clustering.

The worldwide epidemiology of Creutzfeldt-Jakob disease (CJD) is presented from an analysis of 1,435 patients. In the United States, the average annual mortality rate is at least 0.26 deaths per million. Temporal-spatial clustering of cases was not found in the United States, but reports from other countries indicate that such clustering does occur. Fifteen percent of the cases were of the familial type, suggesting a genetic susceptibility to infection. Iatrogenic transmission by corneal transplantation and neurosurgical operations has occurred, and the possibility is raised that previous surgery or preexisting neurological operations has occurred, and the possibility is raised that previous surgery or preexisting neurological disease may be associated with an increased risk of developing CJD. It remains to be determined whether the virus of CJD is maintained only by patient-to-patient transmission, has a zoonotic reservoir such as scrapie, or causes widespread latent infection of man that is occasionally activated.

Adolescent↗
PubMed1978

Quantitative analysis of intracerebral recordings in epilepsia partialis continua.

A 14-year-old female with epilepsia partialis continua was explored stereo-electroencephalographically (SEEG). SEEG, EEG and EMG were recorded synchronously on a 32-channel machine and stored on magnetic tape for off-line analysis. The beginning of the myoclonus was used as trigger for the analysis of the intracerebral activity, which was analysed by averaging. Thereby the generating potentials of the jerks became evident. They had different maxima and latencies relative to the facial and hand muscles: that for m. orbicularis oculi was located in the precentral gyrus, the beginning of its positive deflection was 17 msec earlier than the muscle action potential; that for the thenar muscles had a latency of 24 msec, appeared at first in the premotor cortex and with a slight delay, but with greater amplitude in the corona radiata and capsula interna. Stimulation of the lateral area 4 elicited myoclonus which corresponded to the spontaneous one in distribution and latency. Stimulation of the thalamic VL nucleus had no effect. During deep sleep the frequency of the myoclonus was diminished. REM sleep was preceded and followed by a definite increase of jerking. The pattern, topography and latency of the generating potentials in waking and sleeping were very similar. In this way quantitative evidence of the neocortical origin of Epc is given and therefore a precise delineation of the epileptogenic focus.

Adolescent↗
PubMed1976

Stereo-electroencephalographic exploration and epilepsia partialis continua.

Somatomotor status epilepticus in infatnts consisting of continous repetitive clonic jerks as a sequel of involvment of the sensorimotor area cortex has rarely been explored by multiple depth electrodes. The authors report 3 cases, which were examined stereo-electroencephalographically. Two girls, a 5 year old and a 3 year old showed sustained jerks of one foot with intermittent Jacksonian seizures. Both had a primary epileptogenic focus in the medial prerolandic and postrolandic cortex. The third case, an 8 year old girls, was a borderline case of Kozevnikov epilepsy. All 3 patients were operated on following the stereo-electroencephalographic exploration and the focus was removed. The study shows that it is possible to explore children with depth electrodes and that this exploration is the safest way to localize a focus and, consequently to cure the patient by surgery.

Brain Mapping↗